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High resolution in vivo images of Zebrafish to understand proteinopathies

IGBMC: Caroline Ramspacher and Basile Gurchenkov

Cover image
of Cell Reports #11-10.
June 2015.
Muscle fiber orientation
in the trunk of living
zebrafish larvae
by use of second
harmonic generation.

Developmental Alterations in Heart Biomechanics and Skeletal Muscle Function in Desmin Mutants Suggest an Early Pathological Root for Desminopathies.

Ramspacher C(1), Steed E(1), Boselli F(1), Ferreira R(1), Faggianelli N(1), Roth S(1), Spiegelhalter C(1), Messaddeq N(1), Trinh L(2), Liebling M(3), Chacko N(3), Tessadori F(4), Bakkers J(4), Laporte J(1), Hnia K(1), Vermot J(5).

Cell Rep June 16, 2015

June 5, 2015

Desminopathies belong to a family of muscle disorders called myofibrillar myopathies caused by desmin mutations and leading to protein aggregates in muscle fibers.
The initial pathological steps of desminopathies and the impact of desmin aggregates in the genesis of the disease are unclear.

The team of Julien Vermot at IGBMC developed models of desminopathies in zebrafish in order to understand the origin of the disease.

By implementing live imaging and high resolution microscopy they demonstrated that:

  • desminopathies are associated abnormal alter heart biomechanics and aberrant  calcium dynamics associated with heart contraction,

  • aggregates are more detrimental than desmin loss-of-function,

  • pharmacological inhibition of aggregate formation and Desmin knock-down revert these phenotypes. 


With the help of the team of Jocelyn Laporte (IGBMC) they could show that aggregates are more detrimental than Desmin loss-of-function and that pharmacological inhibition of aggregate formation and Desmin knock-down revert these phenotypes.

Together this alludes to alternative therapeutic approaches and helps in furthering our understanding of the molecular determinants modulating Desmin aggregate formation.

These results are published June 4th 2015 in Cell Reports.

Research funded by AFM (Association française contre les myopathies).

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